Sturge Weber syndrome or encephalotrigeminal angiomatosis is a sporadic neurocutaneous disease characterized by port wine stain color due to a vascular malformation that follows the route of the cranial nerve V (trigeminal) affecting primarily the V1 and V2 branches, in other characteristics that they can present we have the glaucoma, intracranial calcifications and seizure. Its pathogenesis is not yet clarified, is attributed to a genetic component.

Case report: A 46 years old female patient who went to the service of the oral maxillofacial surgery, for evaluation and clinical examination showing purplish macular in the right side of the face, coinciding with the path of V1 and V2 branches of the cranial nerve V without crossing the facial midline, presenting mild paresis, increased volume of the affected area as well as glaucoma previously diagnosed. Intraoral we observed violet maculas stain in the right maxilla, we indicated imaging studies. Management of patients with Sturge–Weber syndrome is focused on treating associated neurologic and ocular abnormalities, hypertrophy of vascular tissue in the affected area.

Conclusion: The clinical management of all diseases including the syndrome is of vital importance to avoid severe complications, which is why we must expand the knowledge of this disease and currently there is insufficient documentation due to the rarity that is presented.

Conflict of interest: None declared.